Bauhammer Jutta, Fiehn Christoph
Abteilung für Innere Medizin/Rheumatologie, ACURA-Rheumazentrum Baden-Baden, Rotenbachtalstr. 5, 76530, Baden-Baden, Deutschland.
Praxis für Rheumatologie, Tätigkeitsschwerpunkt Klinische Immunologie, Medical Center, Baden-Baden, Deutschland.
Z Rheumatol. 2019 Sep;78(7):645-655. doi: 10.1007/s00393-019-0665-0.
Antisynthetase syndromes (ASS) are rare autoimmune diseases. Characteristic is the presence of at least one of the three main symptoms myositis, interstitial lung disease (ILD) and arthritis with possible accompanying symptoms, such as mechanic's hands and feet, Raynaud's disease and/or fever in combination with detection of an aminoacyl-tRNA synthetase antibody in peripheral blood. In addition to myositis, ILD is a frequent and often predominant organ involvement and is responsible for morbidity and mortality. Autoantibodies to 11 aminoacyl-tRNA synthetases are known of which 8 have so far been associated with the clinical manifestation of ASS. The Jo-1 antibody is by far the most frequent one. The antibodies differ in the rate and severity of the main and accompanying symptoms. Treatment with selected immunosuppressive medication depends on the extent and severity of organ involvement. With a 5-year survival rate of approximately 90%, the Jo-1 syndrome has the best prognosis.
抗合成酶综合征（ASS）是罕见的自身免疫性疾病。其特征是存在肌炎、间质性肺病（ILD）和关节炎这三种主要症状中的至少一种，并可能伴有诸如技工手、雷诺病和/或发热等伴随症状，同时外周血中检测到氨酰tRNA合成酶抗体。除肌炎外，ILD是常见且往往占主导的器官受累情况，是发病和死亡的原因。已知针对11种氨酰tRNA合成酶的自身抗体，其中8种迄今已与ASS的临床表现相关。Jo-1抗体是迄今为止最常见的一种。这些抗体在主要症状和伴随症状的发生率及严重程度方面存在差异。选用免疫抑制药物进行治疗取决于器官受累的程度和严重性。Jo-1综合征的5年生存率约为90%，预后最佳。
